Object: Considerable confusion exists in the literature regarding the classification of cerebrovascular malformations and their clinical significance. One example is provided by the atypical developmental venous anomaly (DVA) with arteriovenous shunt, because it remains controversial whether these lesions should be classified as DVAs or as atypical cases of other subtypes of cerebrovascular malformations. The purpose of this study was to clarify the classification of these challenging vascular lesions in an effort to suggest an appropriate diagnosis and management strategy.
Methods: The authors present a series of 15 patients with intracranial vascular malformations that were angiographically classified as atypical DVAs with arteriovenous shunts. This type of vascular malformation shows a fine arterial blush without a distinct nidus and early filling of dilated medullary veins that drain these arterial components during the arterial phase on angiography. Those prominent medullary veins converge toward an enlarged main draining vein, which together form the caput medusae appearance of a typical DVA.
Results: Based on clinical, angiographic, surgical, and histological findings, the authors propose classifying these vascular malformations as a subtype of an arteriovenous malformation (AVM), rather than as a variant of DVA or as a combined vascular malformation.
Conclusions: Correct recognition of this AVM subtype is required for its proper management, and its clinical behavior appears to follow that of a typical AVM. Gamma Knife radiosurgery appears to be a good alternative to resection, although long-term follow-up results require verification.